Hy 90-120 on one page. PLease someone do page 2 and so on to

[Anonymous]

Two patients walk into your office. Listen close.

Patient A has a stroke in motor cortex that lesions UMN tract to central facial n.

Patient B went on a camping trip and has a lesion to the LMN CN VII.

Tell me how each patient will present on physical exams...
Patient A will have CONTRALATERAL, and LOWER QUADRANT paralysis.

Patient B will have same side Bell's Palsy features (can't smile and may drool on affected side)

Another patient comes in weak with signs pointing to anemia. You take a blood smear and whoa! cytopenia...blast cells, reticulocytes, sparse RBCs. And you know this is not autoimmune because it is recent. Hold it...she mentions she had a gonorrheal infection and is on a med. OH YES! OK, so what is the disease, name of the med she is on AND what will be the name of the med you give her as you transfuse bone marrow!?!?!
Chloramphenicol is the drug she is on that caused aplastic anemia. AND you can give cyclosporine or a steroid along with her transfusion. REMEMBER, aplastic anemia has many causes so be careful. Benzene, pregnancy, CMV, HIV, EBV, and autoimmune causes are all to be considered!

You see a female with a blood smear with RBCs small n'round, physical is anemia, hyperbilirubinemia, and abnormal results on the osmotic fragility test. OK OK she has hereditary spherocytosis (so common in clinics). But of course, you need to know:

1) What protein is defective?

2) What is the inheritance pattern?

3) What are the main two complications?

4) Surgical treatment?

5) What do you, an intern prescribe to
) spectrin
2) AD inheritance
3) cholecystitis and aplastic anemia
4) Splenectomy
5) They need folic acid! Them
what drug blocks out enzyme dihydrofolate reductase!!??? ? Trimethoprim blocks it
what abt methotrexate? doesnt that block DHF reductase as well?

Say your pt comes in and you touch both her corneas one at a time with a q-tip, and you note that ONLY the LEFT eye blinks, then which cranial nerve is activated?
Right CN7 (NOT THE LEFT ONE, common mistake)

KEY KEY KEY point. if a patient has no pupillary reaction to light shined on the right side but there is a reaction to light in both eyes, when light is shined on the left. The lesion is what? NOW I change the patient so there is pupil rxn to light on only the right side, when light is shined in either eye. NOW, where is the lesion?
For the first patient, the lesion is the right CN2. For the second, the lesion is left CN3. KNOW IT!

"Don't give ACE inhibitors to a pregnant woman". This is SURE to be on the USMLE Step 1,2,3, but am I breaking a RULE to tell people this VITAL piece of info? I am giving out "exam content" in the sense that I am relaying that IL-5 revs UP IgA and IL-6 (like IL-1) revs up the acute phase response...BUT THESE ARE the BASICS that NBME wants US doctors to master. That is why if I recall from my test a case of a drug overdose and how to treat it, I FEEL COMPELLED to say it on this board in such a way that does not violate copyright laws or "giving out answers". Because....every doctor in the world SHOULD know what drug a person probably took based on his or her symptoms and how to treat them. I encourage everyone to share the concepts after their exams. The NBME should not mind unless I tell everyone that "if you get test version KX-115 then the answer to #1 is B, #2 is A, #3 is E, etc." But to share knowledge that the difference between ALS and multiple sclerosis is that ALS has no sensory deficits, well that is just making everyone wiser and better doctors. What do you guys believe?

Anyhow, let truth reign! Let's say a patient comes into your office at 6:00 pm, my my, and he has vertigo and remarks that he has difficulty with taste and swallowing. Before you give a prescription for antivert, is this a dysfunction of the vestibular apparatus of the inner ear? Or is it a brain stem issue? If it is a brain stem issue, what two nuclei and nerves are involved?
Tricky case. Because vertigo has many causes, note the DIFFICULTY with taste and swallowing. This pushes up the suspicion of a lesion to the nucleus solitarius and ambiguus with nerves 7,9, and 10 also lesioned. AND for the cherry, we see that all the time with a POSTERIOR INFERIOR CEREBELLAR ARTERY stroke which supplies that area! See?

SO, don't just send them home with antivert and a reminder slip for a return 3 month visit!
What MAJOR MAJOR drug other than trimethoprim blocks the loved enzyme dihydrofolate reductase? Methotrexate:

KNOW you often use it for rheumatoid arthritis, hydatiform mole, leukemias and it works its magic in the synthesis phase, stopping thymidine (thymidineless death) and blocks protein synthesis. As I mentioned, I AM NOT REPEATING "EXAM CONTENT" but know that the NBME will give you a picture and ask you to POINT to where methotrexate works its magic. They like doing that. Last year, I wish someone told me just how the NBME likes us to understand stuff. No one told me. Now I want to lift others up.

Koilocytotic atypia of the cervix is associated with which viral inf.?
Uterine enlargement during pregnancy is an example of:
1. hyperplasia
2. hypertrophy
3. hyperplasia&hypertrophy
Ans is 3
Alcoholic with hypocalcemia- treatment.
Answer is Magnesium
Pt with widespread freckles and colonic polypus- 1.what is the diagnosis?
2.she has inc. potential to develop cancer of everything below except:
a)pancreas
b)lung
c)colon
d)breast
e)ovary
f)uterus
Ans 1) colon
2)Puetz jeugers.
DESMOPLASIA - what is it?
excessive fibrous tiss ( CT) formation in stroma of the tumor
What’s the origin of myelin figures that’s seen in late stage of ischemic cell injury?
Damaged membranes
Gardner's synd.- what is it?
BENIGN NEOPLASm OF THE SKULL AND FACIAL BONES> OSTEOMAS..AND GARDNERS SYN IS famil adenomat poliposis +osteoma
What kind of neurons r more susceptible to ischemic injury?
Cerebellum and hippocampal neurons
Which fibers are faster:
1. Myelinated OR unmelinated
2. With small diameter OR large diameter???
1.mylinated.
2.large diameter
What kind of necrosis is caused by immune-mediated vascular damage?
fibrinoid necrosis
Councilman bodies - what is it?
Necrotic deep staining hepatocytes seen in Viral hepatitis,Also in yellow fever.
Hemosiderosis- where the hemosiderine accumulate (which cells).Which tissue or organ damage we can see?
Cells- macrophages
Damage of many tissues and organs but MAINLY :
1.Liver(cirrhosis)
2.Pancreas(fibrosis -> DM)
3.Skin
Lipofuscin most accumulates in these 2 organs in elderly:
1.?
2.?
hepatocytes
Myocardial cells
IL-2 requires which costimulatory factor on T lymhocytes?
1.CD 28
2.B7
3.LFA-1
4.IFN
CD 28 ON T CELL
The osteocytes of one bone connects with each other thro gap junctions in:
1.canaliculi
2.lacuna.
3.howships lacuna.
4.volkmans canal
canaliculi.???????
Pancreatic damage, what enzymes are most affected?1.protease,
2.lipase,
3.elastase
40 y/o patient with BP of 128/80, dec LDL, dec Cholesterol, dec HDL. What is he at risk for?
TANGIER disease (TD)
Why infants with brown fat have more heat?
Thermogenin in brown fat act as uncouplers of oxidative phosphorylation
Dec. ESR- in which diseases?
Polycythemia,Sickle cell anemia,Congestive H failure
The patient with lactase deficiency, what type of diarrhea ?
osmotic diahrea.
Desmin is the marker for...
keratin--------?
desmin- smooth muscle
keratin- epithelial tissue
how do type 2 pneumocytes looks like in EMicroscope.
They are cuboidal- like cells sitting on basal lamina containing lamellar bodies (granules of phospholipid and protein)- but I think this is in LM .What is in EM?
Which side of the membrane Na/k atpase pump you will see
Pump is transmembranal:
ATPase is intracellular
in kidney damage what part of kidney tissue is suspetiable to ischemia?
medulla of kidney mosst commonly effected
matching.
1.acute allergic inf.
2. acute viral inf.
3.acute inflammation.
A.neutophils.
B.lymphocytes.
C.eosinophils
1.acute allergic inf.- > C.eosinophils
2. acute viral inf.- > B.lymphocytes.
3.acute inflammation.- > A.neutophils.
which defect you will see in marfans?
fibrillin.
collagen.
proline/lysine
Fibrillin
Ehlor danlos is a defect of.
fibrillin.
collagen.
proline/lysine
collagen.
waht collagen you will see in EARLYand LATE wound healing?
IN HYPERTROPHIC SCARS Typ 3 collagen then......switch to type 1
and in keloids typ 3....ONLY
DOC for C. difficile
(vancomycin / metro?
Answer is metro
patient being treated with antifungal, and warfarin shows Increasing INR. Which antifungal is culprit?
Gresioflavin
parient being treated with antifungal, and warfarin suddenly develops multi thrombi. Which antifungal is culprit?
Grsioflavin
NSAID have SE at exactly what part of nephron?
aminoglycoside have SE at exactly what part of nephron?
retention of AG in PCT cells disrupt Ca mediated transport.

Is Hydralazine better tolerated by fast or slow acetylators ???
fast acetylators.otherwise SLE like syndrome.
Megdel 8/20/03
Major cause of morality due to DM.(please give your source)
Answer : type-1---->end stage renal d/se
type2--->macrovascular d/se(MI & stroke)
source CMDT.
GAA- repeat mutation- what disease?
Answer : Friedrick Ataxia
Most common:
1.benign soft- tissue tumor
2. malignant ------------
Answer: This Q is from ans. in BSS.They say:
1. leiomyoma
2. malignant fibrous histiocytoma
Which epicondyle in:
1. tennis elbow
2. golfer's elbow
Answer 1. tennis elbow = lateral (remember:LET)
2. golfer's elbow = medial (GEM)
Which part of the spinal cord is mostly susceptible to ischemia? Why?
Answer …Thoracic …..because there is a poor supplementation of the spinal arteries by the radicalar (adamkievitz) arteries in this region
Stasis dermatitis in the legs-why we can see increased pigmentation of skin in this disease
Answer : This Q is from ans in BSS too.
They say- stasis-> hemosiderin deposits in subcutaneous tissue (NO inc. melanin)
In hemochromatosis, Peutz- Jaghers synd., Adisson's disease- hede we have inc. of melanin
stasis--->Haemosiderin--->iron--->stimulates melanogenesis--->inc melanin
What if in the ER a little boy needs blood transfusion-mom says no. Its an ememrgency.What do you do?What if it is not emergency?
If a foreign object is accidentally inhaled, it will likely enter the right lobe, what part of the right lobe if:
1.sitting or standing
2.lying on the right side
1:Lower portion of the right lower lobe.
2ost portion of the right upper lobe.
Or
1.POSTEROBASAL segement of the Right Lower Lobe
2.Posterior segment of the Right Middle or Upper lobe.

Male with ambiguous genetalia, what is the embryological abnormality?
testosterone resistance or 5 alpha reductase def also…mullerian tube dysgenesis
CT of brain shows a defect, which gives loss of sensation to the abdomen?
Answer is post-central gyrus?
branch of middle cerebral artery?
In HY neuro in the sensory homunculi they put "intra abdominal" so I think you are right
Case of man with bloody diarrhea, biopsy shows psuedopolyps.What disease?
Ulcerative colitis
Tumor compressing renal vein, what is result?
1.edema in lower leg,
2.varicocele,
3.esophageal varicies
4.sth.else (what?)
lt. varicocele (if compression of Lt. renal vein)

HUS- what is in the blood tests? Gross pathology changes in
1. Parkinson's
2. Huntington's
3. Wilson's
4. ALS,
5. B12 deficiency
6. Pick's Diz
7. MS
8. Alzheimer's
1. Parkinson's,- substatia nigra
2. Huntington's-caudate nucleus
3. Wilson's-globus pallidus
4. ALS,-anterir horn cells & cortico spinal tracts
5. B12 deficiency- dosal columns & cortiso spinal tracts & spino cerebellar tracts
6. Pick's Diz--? frontal & teporal lobe- with personality changes & progrssive aphasia
7. MS-spino thalamic tracts , dosal colmns & optic nerve & ant horn cells
8. Alzheimer's, depletion of ACH in nucleus basalis ( meynert)
44. 1. Mechanism of action of thipentone
2. side effects
3. what happens at the cellular level
????????
45. Types of bladders: describe and locate the site of lesion.
1. neurogenic
2. automatic
AUTOMATIC BLADDER
If the spinal cord is completely sectioned above the sacral region i.e above the urinary controle centers in the spinal cord ,then initially the patient is in spinal shock and no reflex is present and bladder is atonic.In this stage if the patient is properly is managed i.e timely evacuation is done , gradually the the micturation will develop and it will be automatically evacuated in bladder is FULL.
NEURROGENIC BLADDER
This condition occur in partial spinal cord damage(in case of automatic , it was complete damage)so the facillitatory impulses have influence on the urinary center and inhibitory impulses are blocked.So there is FREQUESNT micturation reflex (in case of autmatic ,timely) with just SMALL AMOUNT of urine(In case of automatic , when bladder is full)
46. Which drug stops translation at the polypeptide stage.?
Macrolides
47. If you stand with one leg on the ground and with the other leg off
the ground and bent at knee which muscle you are stretching.?
Gluteus medius & minimus?
48. gross image of a single large parieto-frontal defect and was asked
as to what is the likely organism that can cause this in AIDS.
Toxo or cryptoccocus????
49. clincal presentation of right parietal lobe infarction.??
non dominent lobe symptoms-negelect of left side,constution apraxia,contralateral homonymous inferior qudrantopia,anosognosia, sensory loss (area 3, 1 ,2) and astereognosis
50. Child with problems reading but no problems with math or social studies. site of Brain lesion?
occipital lobe or splenium???? OR lesion is in wernicke's area , area 22 of temporal lobe and 39 and 40 in parietal lobe(not sure)???
51. Patient with hospital history of 10 admissions for pain but no pathology, what should you do: refer to psych, decr. visits, incr. visits,
admit again?
Admit???
52. what is the most important risk factor for depression?
1:Presence of previous episode
-- After 1st , chances of 2nd episode = 50%
---After 2nd , chances of 3rd eposode =70%
---After 3rd , chances of 4the episode=90%
2:Other most common is psychosocial stressor
--- loss of spouse , beloved one or parents
"The prevalence rate for major depressive disorder appears to be unrelated to ethnicity , education , income and marital status."DSM IV page 341
53. carpal tunnel case, what nerves affected other than median?
????
54. History of patient with horizontal nystagmus, what cranial nerve is involved?
Nerve 8
55. woman could not raise extended leg against resistance, what n injured?
femoral nerve???
56. what increases in the marrow following
hemorrhage...bilirubin,ferritin,erythropoietin,ami nolevulinate
aminolevulinic acid
57. bladder ca ,what defines the malignant
potential...papillary,superficial spreading,adeno...???
superficial spreading
58. PATHWAY OF LDL, WHERE IS CHOLESTOROL RELEASED?

endocytosis mediated by LDL receptor and endosome formation. Then fusion of this endosome with lysosome , LDL is degraded and now cholesterol is released into cell. receptor can go back to the membrane
59. WHAT TYPE OF BOND IS INVOLVED IN SICKLE CELL ANEMIA?
????
60. ohio guy with routine xray showing chest X *** abnormality
1. Dx
2. will U treat (and if Yes, with what; If no, why not?)
Dx.Either Histoplasmosis or Blastomycosis.
In asymptomatic patients no Treatment necessary.
If there is Xray abnormality,,Oral Itraconazole ???????????
61. PCR FUNCTIONS AT WHAT ORGANELLE?
Nucleus since it amplfy DNA
PDGF is the main mitogenic protein for cells of mesenchymal origin in the blood:
a. attracts neutrophils, macrophages, fibroblasts.
b. Is a mitogen
c. Stimulates fibroblasts to secret matrix GAGs and adhesions proteins
d. Stimulates fibroblasts to secret fibroblast-derived collengenase
FGF function:
a. angiogenesis
b. bounded by heparin and the GAG heparan sulfate in the extracellular matrix
c. promote macrophage, fibroblast and endothelial migration into areas of injury
d. is also known to have a role in skeletal muscle development, lung development and hematopoiesis



1.mechanism of blister formation in burns.
2.the main cell acting in the burns blister formation.
3.TB person on treatment with burning pain on leg.vitamin deficiency?
4.alcoholic with ulcers in the mouth.vitamin deficiency?
5.interleukins in hematopoiesis?
6.false positive rate and false negative rate ratio.What will happen to it when we increase sensitivity and decrease specificity.
7.CHF-mechanism of pulmanary hypertension in a patient. Given increase/decrease in hydrostatic pressure and oncotic pressure
8.edema in nephrotic syndrome reason for it. hydrostatic p and oncotic p as choices.
9.Cvs graph with the mechanism of cardiac action.Mark where is s2
10.Action of CCA on the cardiac action potential
11.metformin and the lactic acidosis. given the parameters of ph hco3 and pco2 of a patient with DM. Reason?choices of drugs
12protein folding and the endoplasmic reticulam.
13.adrenal gland histology .. clinical scenario hypertensive on captoprill.
mark the site.
14.adrenal gland histo-mark the site of steroid formation.
15.diarrohrea and ulcers in the intestine. H/o travel+
16.H.pylory and 3-4 q's regarding the relashion ship with ulcers,cancer,treatment and the mechanism of action of the drug for it.
17.insulin and tyrosin kinase actions.
18.hormones and actions(signal transduction mechanisms)
19.nitric oxide and action on vasodialation.
20.action of methorexate
21.X-*** with bamboo spine.
22.aortic aneurism
23.dissecting aorta
24.PDA and the murmur.
25.defctive diaphragm devp and the herniation clinical picture.
26.pleural effusion clinical picture and diag.

Follow
Hi here is the table u r looking for: DISEASE AGGLTN PATTERN OX19 OX2 OXK Epidemic typhus
+++ + -
Endemic typhus
+++ + -
Tick borne spotted ++ ++ -
fever Scrub typhus - - +++

Summery: thus OXK +ve on
schistocytes (similar to TPP)


Rapidly progressive dementia with neurofibrillary tangles (Alzheimer not an option).What is it?
Prion ……crudzfield Jacob disease
What we can see in pancreas biopsy in the cystic fibrosis patient?
Streptokinase-mech. of action.
Blood supply of clitoris.
What cause roseola? How this disease differentiate from others rash-diseases?
Fifth disease…..slap cheek disease fever first then RASh.
Give at least 2 diseases which are caused by unequal crossover.
What i found:
Cri-du-chat
alfa- talasemia
Type of anemia in chronic lymphocytic leukamia.
Auto immune hemolytic anemia
What is the organ secondirily affected in cirrh I would go with spleen, too (by inc. pressure in splenic vessels).Not sure.
osis of the liver?
What is the effect of viral coat on virus ?
Mech. of action of the cholera toxin.Which toxin acts in similar way?
ADP ribosylation--->cAMP increased
ETEC
Alsoooooo same answer as above…increase cAMP.other - e.coli,purtussis,antrax.
When amiloride is mostly used?
Pt on quinidine with atrial fibrillation- what do you give to him?
Postganglionic neurons derive from what?
Neural crest
Phenoxybenzymine- mech. of action.When is used?
alpha blocker (phaechromocytoma)
Inc. of ICP- treatment.
Mech. of action of the drug which is DOC in Bordetella Pertussis inf.
erythromycin, acts at 50s ribosomeinhibiting protein synthsis this way it BLOCK translocation.
Blood in lateral ventricles- where is the source ?(most common)
The longest phase in cell cycle.Give 2 drugs "working" in this phase.
ANSWER Not sure some say G some say S….. need to verify
If its S then these drugs Checked it and according to KPL notes the longest is S phase(7h).G1 is 5h.
So drugs:
hydroxyurea
methotrexate
If G phase then G1
drugs:
1. alkylating agents (cyclophosphamide)
2. Nitrourea

Debeggar 50 qs posted on 8/20/03

which fatty acid necessary in synthesize of arachidonic acid
linoleic
which fatty acid as aspirin-like antiplatelets properties
DOC for exercising asthmatics???
MOA of statins (Reductase not a choice)???
ANSWER is upregulation of LDL receptors
Read below for explanation
MOA: 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors, more commonly known as statins. These agents have been used for primary and secondary prevention of coronary artery disease.
The basic mechanism of action of statins is the reduction of low-density lipoprotein (LDL) cholesterol levels.
Statins also cause minor reductions of triglyceride levels and minor increases in high-density lipoprotein (HDL) cholesterol levels.
The most important adverse effects of statins are elevations of the serum transaminase levels and development of myositis. These adverse effects are more common when statins are used in combination with other medications that inhibit the cytochrome P450 system, such as azole antifungal agents, cimetadine and methotrexate.
The risk for statin-related myositis increases in patients taking gemfibrozil, nicotinic acid or macrolides.
Where in the body is the greatest amount of insulin-inserted-glucose transportation.
Most likely answer is MUSCLEs
why does Aspirin increases breathing rate ?
metabolic acidosis
Where do procainamide and lidocaine work in concert??
fast Na channel
A guy with phimosis and white crap inside, what to give?
Classic triad of Heroin overdose??
miosis,resp deprn,depressed reflexes,pulmonary edema
what happens to Ach effect on the heart when you give NO at the same time??
nothing.reflex tachycardia by NO counteracts Ach effect.
List BP drug contraindicated with Amytryptiline
drugs that decrease NA in synapse (clonidine, guanethidine...)
DOC HTN w/ inc [Na] & reduced [K]
Answer is Ace inhibitors and also LOSARTIN
Is Hydralazine better tolerated by fast or slow acetylators ???
fast acetylators.otherwise SLE like syndrome.
Patient placed on warfarin. Developes severe thrombosis several days later. Most like reason???
inhibition of protein C synthesis occurs fater than inhibition of clotting factors.so unopposed action of clotting factors--thrombosis.

Girl/guy competing in athletic sports and have sign of hirsutism, stunted growth. What is this patient most likely taking?
anabolic steroids
1. What is DOC for mild-moderate Cardiac failure recommended by AMA?
2. whatz its effect on 2.1-preload, 2.2-cardiac contractility, 2.3-postload???
1)ACEI
2)preload--decr,afterload--decr,no effect on contractility.
A patient fall asleep very often and has vivid dream in a 5 minute nap, How to treat him?
amphetamin-narcolepsy
Girl/guy competing in athletic sports and have sign of hirsutism, stunted growth. What is this patient most likely taking?
anabolic steroids
well-built man developes acute abdomen and dies. Autopsy shoes peritonel bleeding.
What was he most likely taking?
anabolic steroids--->liver adenoma--->bleed
aminoglycoside have SE at exactly what part of nephron?
retention of AG in PCT cells disrupt Ca mediated transport
NSAID have SE at exactly what part of nephron?
parient being treated with antifungal, and warfarin suddenly develops multi thrombi. Which antifungal is culprit?
griseofulvin
patient being treated with antifungal, and warfarin shows decreasing INR. Which antifungal is culprit?
Griseofulvin
DOC for C. difficile
(vancomycin / metro?
Answer is metro..
when do U use gold salts in Rx of RA ???
(also give side effects?)
Hypersensitivity/adverse reaction to NSAIDS
DOC for chronic Lyme disease?
Doxy
DOC for craving during alcohol withdrawal ?
Naltrexon
DOC for delirium tremens during alcohol withdrawal ?
Benzodiazepines, chlordiazepoxide is the first choice.
1. Disulfiram inhibits which enzyme specifically?
2. This enzyme has higher affinity for which of the following (or their products)? (methanol / ethanol /ethylene glycol)
aldehyde dehydrogenaseHighest affinity is for ethanol , it is why ethanol is used IV in methanol and ethylene glycol toxicity.
1. MOA ergotamine in Rx of migraine
2. Longterm (insists on this) side effects of ergotamine Rx
vasoconstriction
ID drug causing gingival overgrowths
(give all U know)
What is Rx of this drug-induced gingival overgrowths?
CCB,phenytoin
Nifidepine selectively blocks which channels?
L type Ca channels on vascular smooth muscle.

Mneumonic for cyt p 450
Drugs that increase P-450: Barbara Pheny Greasy Car Reaks on Ethanol
Barbiturates, phenytoin, gryseofulvin, carbamazepine, chronic use of Ethanol.
Drugs that decrease P-450: Quine Iso Somertimes Eats Citrus Grapefruit.
Quinidine, Isoniazid, Sulfonamides, erythromicyn, cimetidine, Grapefruit juice

ANOTHER child walks in with his mom with another immune deficiency. (I keep on with rhymes, he-he-he). Here he is 4 years old, with recurrent otitis media, eczema, and thrombocytopenia from Strep pneumoniae. AND, he bleeds a lot. His IgM is low. Your attending and chief are wondering if you are able to distinguish all these immune def. diseases. Will you get an honors grade(Name disease)? What will you treat with? What is mech that is broken?

He has X-LINKED Wiskott-Aldrich syndrome. This is often confused with the others and Bruton's on exams..hint, hint. But remember the tendency to get attacks from capule bugs like Strep, with otitis, eczema , and BLEEDING. The key is LOW IgM, High IgA,and the bleeding. IgM response curtailed. He is not nearly as bad as SCID case, and you must give him amoxicillin (there are a lot of options here, like you can give ceftriaxone too) plus globins.

This boy has low IgG and presents like WAS syndrome with continued bacterial infections, diarrhea. And you find out this is X-linked too! In the absence of functional Btk, mature B cells expressing surface immunoglobulin and the marker CD19 are few to absent. What disease?

Here is the first immune def. described by Dr. Bruton. So similar to WAS syndrome, but WAS boys will BLEED. OK? Get them straight in your head!!! IT IS HARD!


YET ANOTHER CHILD comes into your peds clinic with an immune def. But this time, the child is hyperreflexic on exam, has abnormal facies, congenital heart disease, hypocalcemia on labs, and increased susceptibility to infections. A radiograph shows he has no thymus! What do you tell your chief? What do you prescribe?


This child has DiGeorge's Disease or thymic aplasia. His 3rd and 4th arch failed to develop. This concept is a favorite of NBME. Including considering marrow transfusion, you must prescribe Calcium salts and Vit D!!!!

ANOTHER, would you believe, child, younger this time, 2 years old walks in, again with recurrent bacterial, fungal, infections. His mom say he suffers often from candida. And you note he has IL-2 def, poss. reticular or ZAP-70 gene def. Your attending walks in and says he will suffer from PCP and Herpes. She (attending) asks you to write a prescription for .... ???? What disease? What med?


This unfortunate child has Severe combined immunodeficiency or SCID. They usually die by age 2 from PCP. You must prophylaxis with TMP-Sulfmethoxazole. Consider IV globin transfusion if counts stay low
Still in your peds rotation, your next patient comes in with recurrent bronchpulmonary, bacterial, neurologic disease, thymus aplasia, telangiectasias, growth retardation, and impaired organ mutation, and is walking funny and waddling. What are you looking at NOW?
you are looking at Ataxia telangiectasia, where both the T and B cells are busted. The alpha fetoprotein levels are always elevated, and they key finding is ATAXIA!


You are beat, but happily this time your patient is not an immune def. case. BUT, you rub your eyes because standing in front of you are 3 answer choices..errr, i mean fraternal triplets (listen I am tired, I have not slept yet)...

LISTEN CLOSE, THEY ALL HAVE systemic symptoms such as weakness, fatigue, malaise, and fever low-grade, two have neck pain, one does not. Physical exam shows hypothyroidism. But here is the concept that comes again again again again:

Child A has hypothyroidism, neck pain, and fever chills and dysphagia

Child B has hypothyroidism, neck pain, and sort of looks a little like he was hyperthyroid last week from history

Child C is shorter and his neck is NOT tender and gets constipation a lot

Child A has ACUTE THYROIDITIS (bacterial) so you must manage aggressively with antibiotics (penicillin G is DOC)

Child B has SUBACUTE THYROIDITIS (viral) so you just give aspirin and return visit. (KEY!!, HYPER, then HYPOthyroid features)

Child C has AUTOIMMUNE THYROIDITIS. This is bad because it is a life-long condition. Treat with levothyroxine.


The clock is approaching 5:30, AND the nurse squeezes in another patient and whaddaknow, he has immune def. with recurrent bacterial sinopulmonary infections. The NBME, er, I mean attending starts pimping you with choices...but you note that the patient is OLDER, LESS SYMPTOMATIC (i.e. less severe disease), and complains of GI symptoms too like diarrhea. What words are coming out of your mouth?

This is the OH SO COMMON IgAD or Immunoglobulin A def. Many stay asymptomatic, IgG and Neutrophil levels could be normal. Give antibiotics....Confused yet? I hope not, I hope I gave you cues to distinguish the diseases.

This is just a day that won't end! Another child, this time let's make it a GIRL, comes in with OF COURSE, an immune deficiency with bacterial and fungal infections. HOWEVER, the NBME has to give up some info (er...I mean the girl's features do I mean..). {This knowledge is good to know for life of course, not just a test}.

Soo...you note that all the immune def. choices are mixing but you see her presenting with lymphadenopathy, hepatosplenomegaly, growth failure, and stigmata of chronic skin infections. Your fellow med student (star student) whispers something about def. w/ phagocytes. TWO distinct hints. AND culture comes back and she has Aspergillus. TELL YOUR ATTENDING WITH CONFID ENCE......!?????
This is Chronic Granulomatous Disease. This is marked by the granulomas (skin stuff) and key words phagocyte def. and Aspergillus infection. Are you getting it all down. YOU HAVE TO IN ORDER TO PASS. All the immune def. will be among answer choices, they differ so slightly. Master them!

You get another child just like the previous case with bacterial infections. BUT, this time you discover there is a defect in microtubules and phagocytics. You see severe gingivitis and oral mucosal ulceration PLUS albinism on the skin. Secondaries: What is the disease, what two bugs eat at you, and what is the first drug you reach for?
Here is Chediak-Higashi disease (not too common). But you get strep and staph infections and you treat with Acyclovir. The KEY to this diagnosis is the mouth stuff and hypopigmentation!

Every single person sitting for USMLE gets one of the Immune def questions, no exception I hear. So, you have a young patient with a gene defective in making myeloperoxidase, thus the cause of his recurrent infections. What cells are weakened, what is the MECHANISM LOST, what is the metal ion in MPO? The ability of the immune cells to engage in respiratory burst is cut off. Myeloperoxidase, MPO, catalyzes the conversion of hydrogen peroxide and chloride ions (Cl) into hypochlorous acid. Hypochlorous acid is 50 times more potent in microbial killing than hydrogen peroxide.
Neutrophils are weakened which contain Fe

You have a patient with a description of allergic rhinitis (some 50 million Americans suffer this, you will see this tested), and he is taking steroids, antihistamines, and pseudoephedrine. He is depressed and wants anti depressants. You pick one from 5 choices and your attending knocks you silly. Which one did you pick that is a no-no?

MAO inhibitors cause hypertensive crisis. You deserved the punch.

patient presents with epigastric symptoms and melena.. You should pick PUD or peptic ulcer disease (this disease is everywhere, like air), BUT there is a secondary! Labs rule out H.Pylori (most common). What is the cause?
Chronic NSAID use. Man, I had to do so many anal exams for this (checking for bleeding with those little Heme cards). They call it the M-3 student consult.

Your patient goes for plastic surgery to look like Michael Jackson and he is given succinylcholine (muscle relaxant). He suffered prolonged respiratory paralysis and muscle paralysis afterwards! What enzyme or mineral is defective? (Hypomagnesium, Hypokalemia, Pseudocholinesterase def)
pseudocholinesterase deficiency. Many causes, but pregnancy, neonates, elderly, burn victims, pesticide poisoning, can be presented by the Boards.

A man comes into your office acting very strange, sticking out his swollen tongue, and complaining of numbness and prickling. He is a vegetarian. What two crucial reactions cannot occur because of the missing diet cofactor?
This is classic triad for Vit B12 deficiency. Homocysteine METHYlation and Methyl malonyl CoA step into TCA cycle is blocked! Ain't that awesome, I mean the knowledge, I feel sorry for the patient though.

You are given a case with a druggie and he has Hepatitis C. Choose and tell me if it is RNA/DNA/SS/DS/Helical/SquareRNA, SS, LINEAR (remember that all RNA viruses are single stranded except Reovirus, AND the letter PCR denote the NON-ENVELOPED VIRUSES or P-Picorna, C-Calic, R-Reo)

Fast! Tell me the ABCs or name three anaerobes and what is name of enzyme lacking which makes them vulnerable to oxidative damage? Actinomyces Bacteroides Clostridium
They are missing catalase. Treat with Clinda above the diaphragm and Metronidazole below the diaphragm!!!

Can you tell me what is the term for the most appearing number amongst a given series of number values? MODE

Slide with megaloblastic anemia, pt looks like a B12 def. Intrinsic factor administered. Patient improves. What disease did he have? (Pick between terminal ileum deficiency and atrophic gastritis) Also, could there be a bug involved? Which oneHe has atrophic gastritis fr. H. Pylori.


Picture like on Webpath of LOBAR Pneumonia. Histo shows encapsulated orgs. Then you see myriads of bact/fungi/viruses as possibilities. What is your first choice?
Strep Pneumoniae!

pic with B1 receptor, which neurotransmitter acts here (Epi, norepi, Ach, Dopamine)?

Now you see a pic of Lung with B2 receptors. Does same neurotransmitter act there? Norepinephrine acts on B1 receptors but NOT B2 receptors (epi does though)

You will be asked questions about Down Syn. Tell me:

What is the organ most commonly affected (although Down's hits all systems)?

What cancer is associated?

What hormone do you often treat them with?

Is alpha feto protein low or high at 14 week gest?

Cardiac (e.g. VSD)

Cancer is ALL

Hormone is thyroid hormone

Alpha fetoprotein is low in testing

An African American male comes into your office with signs of very very mild anemia, almost no symptoms, a little jaundice. His main complaint--a UTI. Your senior hints this is the most common enzyme pathology. A smear shows Heinz bodies (review please). Now your senior starts a pimping away.

1) What is his disease?

2) Why is it so prevalent?

3) What does the enzyme catalyze? What is the end product?

4) You grab some sulfamide and nitrofurantoin to treat his Urinary Tract Infection and your attending smacks you on the other side of the face that she missed before. Why was she so upset with you?

1) G6PD Deficiency

2) It confers protection against malaria

3) The G6PD enzyme catalyzes the oxidation of glucose-6-phosphate to 6-phosphogluconate while concomitantly reducing the oxidized form of nicotinamide adenine dinucleotide phosphate (NADP+) to nicotinamide adenine dinucleotide phosphate (NADPH). NADPH, a required cofactor in many biosynthetic reactions, maintains glutathione in its reduced form. RBCs need NADPH to protect itself against oxidative stresses. (Long winded explanation, but you have to know it., sorry).

4) You cannot give an oxidizing agent like primaquine, choroquine, or a sulfa drug, or nitrofurantoin to a patient with G6PD def. Their RBC will hemolyse and you will lose your license and your attending will lose her's and your hospital will close and turn into an apartment complex.

[kris2c]

where are these q's from ?